FBN1 and systemic sclerosis: FBN1 expression is increased in scleroderma skin [55], which is unusually degradation-susceptible [56], and the presence of anti-fibrillin-1 antibodies in SSc is thought to activate fibroblasts and stimulate the release of TGF-β [57,58], all supportive of FBN1′s potential role in scleroderma pathogenesis.